Langerhans cell granulomatosis


Authors: J. Homolka 1, 2;  I. Haškovcová 3
Authors‘ workplace: I. klinika tuberkulózy a respiračních nemocí 1. lékařské fakulty UK a VFN Praha, přednosta prof. MU Dr. Jiří Homolka, DrSc. 1;  Pneumologická klinika 1. lékařské fakulty UK a FTN Praha, přednosta prof. MU Dr. Jiří Homolka, DrSc. 2;  Ústav patologie 1. lékařské fakulty UK a VFN Praha, přednosta prof. MU Dr. Ctibor Povýšil, DrSc. 3
Published in: Vnitř Lék 2010; 56(Supplementum 2): 74-75
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

Langerhans cell granulomatosis is a rare disease of unknown aetiology and characterized by an infiltration of alveolar septa with activated Langerhans cells and eosinophylic granulocytes. Accumulation of these cells leads to a development of multiple cystic formations. The involvement of the lungs is usually present on its own, more rarely it is seen as part of a systemic disease involving skeleton or posterior pituitary. The disease prognosis in adult patients is usually good, 80% of patients achieve remission or stabilization of the disease either spontaneously or following a corticosteroid therapy, 10– 20% of cases progress to respiratory failure.

Key words:
Langerhans cell granulomatosis –  diagnostics –  BAL –  treatment


Sources

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4. Skácel Z, Marel M, Vraštilová P et al. Histiocytóza z Langerhansových buněk. Stud Pneumol Phthiseol 2000; 60: 150– 156.

5. Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J 2006; 27: 1272– 1285.

6. Yağci B, Varan A, Cağlar M et al. Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center. Pediatr Hematol Oncol 2008; 25: 399– 408.

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Labels
Diabetology Endocrinology Internal medicine

Article was published in

Internal Medicine

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